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PubMed1990

[Myopathy in acromegaly. Report of two cases].

Acromegaly is often associated with neuromuscular disorders. Most of them are caused by compression of nerves with hypertrophic bone and soft tissues or complications of diabetes mellitus. Myopathy has rarely been reported in the Japanese literature. We report two cases with myopathy out of 14 cases of acromegaly. Case 1 is a 62-year-old woman who developed muscle weakness and atrophy in the shoulder girdle, pelvic girdle and femoral regions after a 10-year history of acromegaly. She showed positive Gowers' sign and normal DTRs. Basal growth hormone (GH) level in plasma was 1076 ng/ml. Electromyograms (EMG) obtained from the deltoid and rectus femoris muscles revealed typical myopathic abnormalities; an excess of small-amplitude, short-duration, polyphasic motor unit potentials. Histological examinations of the rectus femoris muscle showed diffuse atrophy of both type I and type II fibers. She also had bilateral carpal tunnel syndrome and bilateral tarsal tunnel syndrome, which were confirmed by nerve conduction studies of median nerves and posterior tibial nerves. A cranial computed tomography (CT) scan demonstrated sellar mass with suprasellar extension. She underwent transsphenoidal adenomectomy and radiation therapy. GH level lowered to 29 ng/ml, however, myopathy remained unchanged for 3 years after the surgery. Case 2 is a 38-year-old woman who had undergone partial removal of a pituitary adenoma 9 years after the onset of acromegaly. Basal GH level in plasma before the surgery had been 1694 ng/ml and was still high after the surgery (100-505 ng/ml). The patient developed proximal muscle weakness and atrophy 4 years after the surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

Acromegaly↗
PubMed1987

[Psychopathologic symptoms and personality traits in patients with florid acromegaly].

Important technical and clinical progress has been achieved in neuroendocrinological diagnostic investigations in recent years. Patients with active acromegaly and without a total loss of pituitary anterior lobe function can now be defined. With the help of neuroradiological investigations the intra-, supra- and parasellar extent of the adenoma can be determined. For the following study we selected patients with active acromegaly without a significant reduction of the other pituitary anterior lobe functions and with intrasellar adenomas. 31 patients with active acromegaly were examined before the operation. These patients have uniform psychopathological symptoms including loss of drive, affective disorders such as dejection, brooding, and irritability, and increase in appetite and loss of libido. The psychopathological symptoms are dependent neither on the level of the increased growth hormone nor on hyperprolactinemia. The higher cortical functions are intact. No disorders of intelligence or memory can be found. Disturbances of mental functions are manifested as disorders of concentration and fatigue. Personality traits were strikingly uniform. Their personalities are characterized by conscientiousness, reliability and industriousness. Some patients are also anxious and lacking in self-confidence. The personality traits of the patients become more pronounced as a result of the illness. The continuous development of the patients corresponds to the personality traits. Changes in personality, in particular those caused by organic brain disease were not found. Because we applied strict criteria to the selection of our patients we conclude that the uniform psychopathological symptoms and the uniform personality traits of the patients are an essential element of the clinical picture of active acromegaly.

Acromegaly↗
PubMed1989

Primary hyperthyroidism occurring in association with acromegaly.

Two patients with acromegaly who also developed primary hyperthyroidism are described. One case presented de novo with Graves' disease and acromegaly and the other developed hyperthyroidism several years after the diagnosis of acromegaly had been made. No associated adenomas could be demonstrated. The association between acromegaly and thyroid disease is discussed.

Acromegaly↗
PubMed1987

Acromegalic heart disease: influence of treatment of the acromegaly on the heart.

In a series of 256 patients with acromegaly, 10 had evidence of heart disease for which no explanation apart from the acromegaly could be found. Heart disease presented with effort dyspnoea, cardiac failure, palpitation, ECG changes or cardiomegaly. Initial chest radiographs showed cardiac enlargement in seven patients. Electrocardiograms were abnormal in nine patients with repolarisation disorders or intraventricular conduction defects. Rhythm disturbances were found in six. Echocardiograms were performed on six patients; all were abnormal showing left ventricular hypertrophy or impaired function. In five patients radionuclide ventriculography was also performed. Cardiac catheterisation was undertaken on seven patients; all showed either hypertrophy or dilatation of the left ventricle. Coronary arteries were widely dilated in two patients and in another there was dilation of the proximal segment only. In six of the 10 patients, acromegaly was cured by transsphenoidal surgery. This resulted in limited improvement of cardiac function in two patients only. Of the four patients who were not cured, three died and one was lost to the study. Four patients in total died and autopsies were obtained in two: one showed changes suggesting myocarditis and the other diffuse fibrosis. It is concluded that acromegaly may infrequently lead to heart disease, and that if recognised at an early stage progression may, in a proportion of patients, be arrested by successful treatment.

Acromegaly↗
PubMed1988

Electron microscopical morphometry of pituitary adenomas. Comparison of tumours in acromegaly and hyperprolactinemia.

By electron microscopical morphometry (point counting method) 4 groups of adenomas were compared in order to identify the source of prolactin in hyperprolactinemia. The 4 types of adenomas were characterized by the following features: Group I: acromegaly without hyperprolactinemia (GH positive and PRL negative on the immunohistochemical level); Group II: acromegaly with hyperprolactinemia and/or galactorrhea (GH positive and PRL positive on the immunohistochemical level); Group III: adenomas with hyperprolactinemia without acromegaly (GH negative and PRL positive on the immunohistochemical level); Group IV: adenomas with hyperprolactinemia without acromegaly (GH and PRL negative on the immunohistochemical level). Morphometry was performed in order to analyse the relative amount of the following cellular structures: nuclei, nucleoli, rough endoplasmic reticulum, Golgi fields, immature secretory granules, mature secretory granules, lysosomes, mitochondria, unorganized cytoplasm, and cellular membranes. Significant differences (Student t-test, Wilcoxon test; 2 p less than 0.05) were found for the following compartments: rough endoplasmic reticulum and Golgi fields in group III had significantly larger volumes than in group IV. The volume of secretory granules of group II and group IV was larger than of group III. The volume of mitochondria of group IV was larger than in group I, and it was larger in group I and group III than in group II. Despite these differences a distinctive morphometrical pattern of the different subgroups could not be established. The quantitative data are valuable for interpretation of high or low functional activity but not for differential diagnoses. Therefore, if only the source of PRL in hyperprolactinemia has to be identified, immunocytochemistry is the best and simpler method.(ABSTRACT TRUNCATED AT 250 WORDS)

Acromegaly↗
PubMed1987

Acromegaly update--etiology, diagnosis and management.

Acromegaly is a disease with unique clinical manifestations. Its confirmatory diagnosis, however, requires basal and dynamic tests of growth hormone secretion. The measurement of circulating levels of somatomedin C has been a valuable addition to the diagnostic armamentarium. We review the etiology of acromegaly, with particular reference to the different histochemical and ultrastructural forms of somatotropic adenomas and their respective clinical behaviors. Ectopic sources of growth hormone-releasing hormone and of growth hormone itself are now well-recognized, though unusual, causes of acromegaly. The treatment of acromegaly is often problematic and far from uniformly successful. Initial enthusiasm for the results of surgical treatment has now been tempered by reports of increasing rates of recurrence on long-term follow-up. The roles of irradiation and pharmacotherapy are reviewed with particular emphasis on the use of bromocriptine, which has added a new dimension to the control of the somatic and metabolic manifestations of hypersomatotropism. Studies have been done recently using a long-acting somatostatin analog with encouraging results.

Acromegaly↗
PubMed1986

[Association of acromegaly and a cerebral arterial aneurysm disclosed by a subarachnoid hemorrhage].

The association of acromegaly and cerebral aneurysm is an infrequent clinical finding, being few the cases reported in literature. A major incidence of cerebral aneurysm in patients with other pituitary adenoma and cerebral tumour has been described, in relation to the general population. We have studied a male patient, aged 37 years old, with an acromegaly disease initiated 5 years before, that came to our hospital because of subarachnoid hemorrhage symptomatology. A right middle cerebral aneurysm was found in surgery. Hormonal studies confirmed a high basal Growth Hormone (64 ng/ml) that was not suppressed during an Oral Glucose Tolerance Test (75 g). A triple stimulus test (Insulin, TRH, LHRH) demonstrated non-response of GH to TRH and hypoglycemia others hormones were normal. Radiological studies were suggestive of Acromegaly disease. Computed axial tomography showed an intra-sellar tumour. The patient was operated (Transsphenoidal surgery) and a GH pituitary secreting tumour found, without suprasellar extension. The clinical significance and importance of the coexistence of the Acromegaly and Cerebral Aneurysm is discussed, with a review of hypothesis implicated.

Acromegaly↗
PubMed1983

[Acromegaly, clinical expression of the production of growth hormone releasing factor in pancreatic tumors].

In exceptional cases, acromegaly develops as the clinical expression of an ectopic secretion of Growth Hormone (GH) or Growth Hormone-Releasing Factor (GRF), tumorous in origin. In the present report, we describe an instance of acromegaly caused by the secretion of GRF from a voluminous pancreatic tumor. The resection of this tumor resulted in a temporary disappearance of the biological and clinical symptoms of acromegaly, which then reappeared in conjunction with a rise in plasma GRF. From this pancreatic tumor, substances displaying a potent GRF activity were isolated and characterized. Amino acid analyses revealed that they were related to 3 peptides containing respectively 44, 40 and 37 aminoacids. The largest (hp GRF (1-44)-NH2) referred as hp GRF or somatocrinin is considered to be the primary molecule. The pancreatic tumor was multisecreting as proved by high plasma levels of somatostatin, pancreatic polypeptide and glucagon, normalized after the tumor removal, taken together with the immunocytochemical demonstration of the presence of these peptides in the tissue and with the isolation of somatostatin. In contrast hypercalcemia associated with an elevated plasma level of IR-PTH was unmodified by tumor removal. Diagnosis of acromegaly as ectopic endocrine syndrome will probably be facilitated by plasma GRF radioimmunoassay, as a result of production of anti synthetic GRF antibodies.

Acromegaly↗
PubMed1982

Diagnosis of acromegaly on cranial computed tomography.

The characteristic features of acromegaly as demonstrated on cranial computed tomography (CT) are described. These features relate to the increased soft tissue bulk and overpneumatization of sinuses and air cells, as well as the generalized bony overgrowth associated with high growth hormone (GH) levels. In the assessment of these features, the computer window settings must be adjusted accordingly for separate visualization of soft tissues, calvarial bones and cerebral tissue on the scan images. We found positive features in CT scans of 13 patients with established acromegaly and were able to suggest the diagnosis prospectively in 2 additional cases. The finding of an enlarged pituitary fossa should lead the radiologist to search carefully for the ancillary signs of acromegaly on the scans, thus confirming the diagnosis. When an enlarged sella turcica or an enhancing intrasellar mass is not demonstrated, the recognition of the other features on cranial CT scans may be important in suggesting the diagnosis of acromegaly to the clinician.

Acromegaly↗
PubMed1994

Normal insulin-like growth factor-I in an elderly woman with acromegaly and medical illness.

Insulin-like growth factor-I (IGF-I) has been advocated as a simple and reliable test for confirming the diagnosis of acromegaly and following a patient's response to treatment. We describe an elderly woman admitted to the hospital with altered mental status, congestive heart failure, and arthritis who was noted to have clear features of acromegaly. An IGF-I level was in the normal range, while results of an oral glucose tolerance were diagnostic of acromegaly. As her medical condition, mobility, and nutrition improved, her IGF-I level increased above the normal range and continued to rise when she was seen as an outpatient. Therefore, illness, physical activity, nutritional status, and advanced age must be considered when interpreting IGF-I levels in patients with known or suspected acromegaly.

Acromegaly↗
PubMed1994

Acromegaly and transsphenoidal hypophysectomy: a case report.

Anterior pituitary tumors account for nearly 18% of all intracranial tumors. Pituitary adenomas that cause hypersecretion of growth hormone lead to acromegaly in adults. Patients with acromegaly may present unique problems for the anesthetist because of the overgrowth of airway soft tissues; a difficult mask ventilation and challenging intubation can be expected. A careful preoperative assessment of the patient's airway is essential, and an awake oral or fiberoptic bronchoscopy may be necessary. Postoperatively, these patients are at risk for developing airway problems and diabetes insipidus; therefore, they warrant careful observation. A 42-year-old, 75-kg, ASA physical status III, white male presented 8 months after suffering a head injury in which he was knocked unconscious for approximately 3 minutes. He began experiencing severe headaches, visual changes, and a marked increase in the size of his hands and feet. Four months before admission, he underwent bilateral carpal tunnel repairs. The patient was diagnosed with acromegaly after an extensive endocrine and neurosurgical evaluation. This is a case report of a patient with acromegaly who underwent an elective transsphenoidal hypophysectomy.

Acromegaly↗
PubMed1993

An audit of outcome of treatment in acromegaly.

In order to determine whether acromegaly is still associated with increased mortality, a hospital case note review of all patients with acromegaly followed up in Stoke-on-Trent since 1967 was carried out. Of 79 subjects identified, 51 are alive and being monitored and 28 have died. Mortality was compared to the general population by life table analysis. Secretion of growth hormone was assessed and compared in dead and alive patients. The effect of diabetes, hypertension, and growth hormone secretion on long-term outcome was assessed. Acromegaly is still associated with increased mortality, with an overall ratio of observed to expected deaths equal to 2.68 (95% C.I. 1.8-3.9; p < 0.001), but the survival of 31 (39%) patients whose growth hormone level had been reduced to below 5 mU/l was equal to that of the general population (O/E = 1.42; 95% C.I. 0.46-3.31: p > 0.05). The dead patients had had significantly higher growth hormone levels than those still alive, but mortality did not appear to be influenced by diabetes or hypertension. The cause of death was vascular in 57% of cases. Growth hormone hypersecretion is still associated with excess mortality in acromegaly. The present study suggests that the therapeutic objective should be to lower average daytime growth hormone levels to less than 5 mU/l. There is need for a large study to compare different modes of treatment in terms of their effect on growth hormone secretion and on long-term outcome.

Acromegaly↗
PubMed1993

[A case of left ventricular aneurysm associated with acromegaly].

We performed a left ventricular aneurysmectomy for a left ventricular aneurysm associated with acromegaly in a 62-year-old man. Bromocriptine mesilate was administered to suppress high growth hormone (GH) levels, and there were no complications during or after the operation. The majority of patients with acromegaly die of cardiovascular diseases such as cardiomegaly, hypertension, arteriosclerosis of the coronary arteries, congestive heart failure, valvular disease, and arrhythmia. Ventricular aneurysm is a rare complication of acromegaly. In order to safely operate, it is important to control serum GH levels and pay special attention to the well-known complications of acromegaly such as diabetes mellitus and hypertension.

Acromegaly↗
PubMed1995

[Surgical treatment of familial acromegaly].

The annual incidence of the acromegaly is three cases per million, and that of the familial acromegaly is even far rarer. The number of familial acromegaly reported in the medical literature of all the world is fewer than 10 cases per year, and up till now none of such a patient has been reported in China. All the three cases of familial acromegaly reported here were from the same family, and showed typical clinical manifestations. They presented the history of acral enlargement, headache, amenorrhoea, elevated serum GH level, enlargement and erosion of pituitary fossa in X-ray and CT scan, and characteristic immunohistochemical findings of GH-secreting pituitary adenoma. All of them underwent successful transsphynoidal total removal of the pituitary adenoma, and recovered well by clinical, endocrine, and pathological criteria. An extended family tree of 65 members excluded consanguinous marriage and suggested that the mode of inheritance was irregular autosomal dominant.

Acromegaly↗
PubMed1977

Failure of selective pituitary adenomectomy to cure acromegaly.

Complete selective pituitary adenomectomy for acromegaly may fail to cure the underlying endocrinopathy. A 40-year-old man who had acromegaly of ten-years duration was initially treated with pituitary irradiation eight years prior to surgery. Three years after irradiation his mean GH concentration decreased from 53 to 9.0 ng/ml, then subsequently increased, and was associated with development of severe hyperglycemia. A transsphenoidal microdissection was performed, and a discrete pituitary adenoma was visualized and totally removed at surgery. GH concentrations decreased from 46 to 5.0 ng/ml one week after surgery and to 3.0 ng/ml 10 months after surgery. Despite improvement postoperatively, the patient still demonstrated abnormal GH dynamics and responses to the GTT, abnormal nocturnal and random GH sampling, and abnormal responses to the L-dopa, apomorphine, and TRH stimulation tests. Acromegaly could be caused by either hypothalamic hyperfunction, an autonomous pituitary tumor, or both; but the latter would not explain the results obtained in this patient. This suggests that reduction of GH to normal concentrations following selective pituitary adenomectomy is not synonomous with curing acromegaly.

Acromegaly↗
PubMed1996

[A case of acromegaly associated with variegated spinal disorders].

A case of acromegaly associated with variegated spinal disorders was reported. The spinal disorders were multiple cervical disc herniations, spinal epidural cavernous angioma, multiple ossification of the spinal ligament and lumbar canal stenosis. A 51-year-old woman with acromegaly, complaining of disturbances of delicate hand movement and gate, consulted our department. Her past history included diabetes mellitus, hypertension and progressing enlargement of her extremities. Serum growth hormone level was 65.7 ng/ml and somatomedin-c level was 746 ng/ml. Brain MRI showed a pituitary tumor extending to the right cavernous sinus. Cervical MRI revealed disc herniations at C5/6 and C6/7. Thoracic MRI revealed osteoporosis, ossification of the posterior longitudinal ligament and multiple ossification of yellow ligament. Lumbar MRI disclosed ossification of yellow ligament and canal stenosis. Anterior fusion of C5-C7 and an intracapsular removal of the pituitary tumor were performed. Its pathology was that of eosinophilic adenoma. After 3 months, she suffered from paraparesis. On repeating MRI examination with Gd-DTPA, a spinal epidural mass was found at T4. Under laminectomy of Th3-5 and Th8-11, the epidural mass and ossified yellow ligament were removed. The epidural mass was cavernous angioma. She was able to walk without any assistance. An association of spinal canal stenosis with acromegaly is well known. But the association of disc herniation and with the ossification of spinal ligaments is rather rare in the literature. Spinal epidural cavernous angioma is very rare. We discussed the etiological aspects and the management of spinal disorders with acromegaly.

Acromegaly↗
PubMed1996

[Heart disease in acromegaly. Study of 27 patients].

BACKGROUND: The patients with acromegaly have an increased propensity to develop cardiovascular complications. The aim of this study was to evaluate the most relevant clinic, echocardiographic and Holter recording findings in acromegaly and its correlation with the growth hormone (GH) levels and duration of the disease. PATIENTS AND METHODS: 27 patients with acromegaly were evaluated. The basal levels of GH and insulin-like growth factor 1 (IGF-1) were measured at the time of cardiological study and compared with the spontaneous GH secretion during 12-24 hours previously studied. Echocardiogram was made using complete M-mode, two dimensional and spectral Doppler ultrasound mechanical system. Additionally an standard EKG and a Holter recording were performed during 24 hours. RESULTS: There was a strong correlation between basal levels of GH and integrated concentration of GH during the study of spontaneous secretion (r = 0.92; p < 0.01). The prevalence of symptomatic cardiovascular complications in this study was 49%, including 3 patients who died for cardiovascular events. The echocardiographic study showed a high prevalence of left ventricular hypertrophy (66.7%), diastolic dysfunction (63%) and impaired systolic function (18.5%). There was no correlation between GH and IGF-1 levels and echocardiographic findings. Conversely the degree of hypertrophy was related to the duration of the disease and the presence of arterial hypertension. The diastolic function only was related with the presence of hypertension. We found supraventricular arrhythmic events and malignant premature ventricular complexes in 6 and 6 patients, respectively. The EKG showed a high fiability to detect ventricular hypertrophy but no rhythm events. CONCLUSIONS: The high prevalence of cardiovascular complications in acromegaly, most of them asymptomatic, and its clinical relevance, advise the necessity to perform systematic cardiovascular evaluation in these patients.

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PubMed1996

[Cutaneous manifestations of acromegaly: 4 cases].

INTRODUCTION: Some skin changes in acromegaly belong to the classical dysmorphic syndrome. Furthermore, other minor skin changes can be associated. OBSERVATIONS: We report 3 patients consulting in the department of Dermatology for various skin changes that have lead to the diagnosis of acromegaly. However these patients have also typical dysmorphic syndrome that they have failed to recognize. The fourth patient associated a cutaneous mastocytosis and acromegaly, as reported only once in literature. The evolution of the minor skin changes was parallel with the endocrinopathy. Moreover in one case, they have preceded the recurrence of the acromegaly. COMMENTS: Our observations demonstrate the importance of the minor skin changes that lead the patient to consult more than the dysmorphic syndrome. Moreover these changes that depend of growth hormone and other associated growth factors can be evolutive signs for endocrinopathy.

Acromegaly↗